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Exploring the molecular basis for variability among patients with Becker muscular dystrophy: dystrophin gene and protein studies.
TLDR
P phenotypic variability among patients with similar mutations suggests that epigenetic and/or environmental factors play an important role in determining the clinical progression of Becker muscular dystrophy. Expand
Serum creatine phosphokinase. Activity in progressive muscular dystrophy and neuromuscular diseases.
TLDR
The present communication is concerned with the determination of creatine phosphokinase and aldolase activities in the sera of patients with progressive muscular dystrophy and neuromuscular diseases. Expand
Muscle-specific calpain, p94, is degraded by autolysis immediately after translation, resulting in disappearance from muscle.
TLDR
In vitro translation experiments support the idea that p94 shows nuclear localization when expressed in COS cells, and results indicate the p94-specific IS2 region plays an important role in this degradation. Expand
Absence of extraocular muscle pathology in Duchenne's muscular dystrophy: role for calcium homeostasis in extraocular muscle sparing
TLDR
It is shown that dystrophin deficiency does not result in myonecrosis or pathologically elevated levels of intracellular calcium ([Ca2+]i) in EOM, and modulating levels of calcium homeostasis in muscle may be of potential therapeutic use in DMD. Expand
Molecular cloning and functional analysis of three subunits of yeast proteasome.
TLDR
Results indicate that cloned proteasome subunits having similar sequences to the yeast Y13 subunit are structural, but not catalytic, components of proteasomes. Expand
Abnormal localization of laminin subunits in muscular dystrophies
TLDR
In FCMD muscle, a significant reduction of laminin M (merosin; a striated muscle specific basal lamina-associated protein) is found with approximately 26% of levels seen in controls by quantitative immunofluorescence, which may implicate a primary or central role for the basalLamina in FC MD muscle. Expand
An ATP-dependent protease and ingensin, the multicatalytic proteinase, in K562 cells.
TLDR
The results suggest that the inactivation of ingensin occurring on preincubation is not irreversible, and the ATP-dependent protease investigated is identical to the high-molecular-mass protease, ingens in human erythroleukemia, previously reported by us. Expand
Identification of a third ubiquitous calpain species--chicken muscle expresses four distinct calpains.
TLDR
This study identified three kinds of cDNAs encoding distinct chicken calpain large subunits that were highly similar to the mammalian mu-type and p94, respectively, and designated them 'mu/m-calpain', because its sequence and Ca(2+)-sensitivity lie between mu- and m-types. Expand
Pathophysiology of muscle fiber necrosis induced by bupivacaine hydrochloride (Marcaine)
TLDR
Since the sequence of fiber breakdown induced by BPVC administration was similar to that of progressive muscular dystrophy, this chemical will be one of the most useful tools for studying the pathophysiology of fiber necrosis and regeneration in diseased muscle. Expand
Quadriceps myopathy: Forme fruste of Becker muscular dystrophy
TLDR
It is concluded that the syndrome called quadriceps myopathy includes a group of forme fruste Becker muscular dystrophy, and dystrophin of an abnormal molecular mass was visualized in muscle cryosections as “patchy” or discontinuous immunostaining at the surface membrane of the muscle fibers. Expand
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